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Natural history of polycythemia vera and essential thrombocythemia presenting with splanchnic vein thrombosis.
Annals of Hematology ( IF 3.5 ) Pub Date : 2020-02-22 , DOI: 10.1007/s00277-020-03965-z
Alberto Alvarez-Larrán 1 , Arturo Pereira 2 , Marta Magaz 3 , Juan Carlos Hernández-Boluda 4 , Marta Garrote 1 , Beatriz Cuevas 5 , Francisca Ferrer-Marín 6 , M Teresa Gómez-Casares 7 , Valentín García-Gutiérrez 8 , M Isabel Mata-Vázquez 9 , Fanny Turon 3 , Virginia Hernandez-Gea 3 , Eduardo Arellano-Rodrigo 10 , Francisco Cervantes 1 , Juan Carlos García-Pagán 3 ,
Affiliation  

Patients with polycythemia vera (PV) or essential thrombocythemia (ET) presenting with splanchnic vein thrombosis (SVT) might have a specific clinico-biological profile. To investigate this hypothesis, 3705 PV/ET patients from three national registers, 118 of them presenting with SVT, were reviewed. After correction for age and sex, PV/ET patients with SVT showed an increased risk of death (HR 2.47, 95% CI 1.5-4.01, p < 0.001), venous thrombosis (IRR 3.4, 95%CI 2.1-5.5, p < 0.001), major bleeding (IRR 3.6, 95%CI 2.3-5.5, p < 0.001), and second cancer (IRR 2.37, 95%CI 1.4-4.1, p = 0.002). No case of acute leukemia was documented among patients with PV/ET presenting with SVT and seven of them (6%) progressed to myelofibrosis. SVT was not associated with lower risk of MF after correction by age and sex. Patients with SVT more frequently died from complications related to hepatic disease, major bleeding, or second cancer, resulting in a 5-year reduction of age- and sex-adjusted median survival. In conclusion, PV and ET patients presenting with SVT have shorter survival than patients with PV and ET of the same age and sex. This excess mortality is related to liver disease, major bleeding, and second cancer rather than to the natural evolution of the MPN.

中文翻译:

真性红细胞增多症和原发性红细胞增多症的自然病史伴有内脏静脉血栓形成。

伴有内脏静脉血栓形成(SVT)的真性红细胞增多症(PV)或原发性血小板增多症(ET)的患者可能具有特定的临床生物学特征。为了研究该假设,对来自三个国家登记处的3705名PV / ET患者进行了回顾,其中118名出现SVT。校正年龄和性别后,SVT的PV / ET患者的死亡风险增加(HR 2.47,95%CI 1.5-4.01,p <0.001),静脉血栓形成(IRR 3.4,95%CI 2.1-5.5,p < 0.001),大出血(IRR 3.6,95%CI 2.3-5.5,p <0.001)和第二癌(IRR 2.37,95%CI 1.4-4.1,p = 0.002)。PV / ET合并SVT的患者中无急性白血病病例,其中7例(6%)进展为骨髓纤维化。按年龄和性别校正后,SVT与较低的MF风险无关。SVT患者死于与肝病,大出血或第二次癌症相关的并发症,导致按年龄和性别调整的中位生存期缩短了5年。总之,伴有SVT的PV和ET患者比同年龄和性别的PV和ET患者生存期短。这种过高的死亡率与肝脏疾病,大出血和第二次癌症有关,而不是与MPN的自然进化有关。
更新日期:2020-02-23
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